PNP Management 3 Exam #1

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1
5 Hepatoviruses
-Hepatitis A
-Hepatitis B
-Hepatitis C
-Hepatitis D
-Hepatitis E
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Hepatitis A
-Causes primary infections in the liver
-Transmitted easily in homes and childcare centers through person-to-person contact via fecal matter found on contaminated surfaces
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When can the Hep A vaccine be given prophylactically?
12 to 23 months
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Risk Factors: Hepatitis A
-Personal contact with an infected individual:
can be spread to adults easily because children <6yrs are asymptomatic
-International travel
-Food-borne outbreaks
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Clinical Manifestations: Hepatitis A
-Older children often present with similar symptoms to adults
-Nausea
-Vomiting
-Anorexia
-Fever
-Right upper quadrant pain (dull)
-Jaundice in the icteric phase
-Hepatomegaly
-Splenic tenderness
-Adenopathy
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Clinical Manifestations Continued: Hepatitis A
-Children may also have darkened urine and clay-colored stools
-Infants may have diarrhea, while constipation is more common in older children
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Clinical Phases: Hepatitis A
-Preicteric phase:
acute febrile illness
malaise
nausea
vomiting
digestive complaints
-Icteric phase:
jaundice
dark urine
clay-colored stools
poor weight gain
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Management: Hepatitis A
-Supportive teatment
-Hep A vaccine or immunoglobulin for post exposure prophylaxis within 14 days of exposure
-Immoglobulin can be used for kids <2 years
-Immunoglobulin can be administered in some high exposure cases or prophylactically, in conjunction with HAV vaccine for those traveling internationally
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Hepatitis A Phrophylaxis Indications
-Household contacts and sexual partners of known cases
-Oersons accidently inoculated with a contaminated needle
-Newborn infants of infected, jaundiced mothers
-People with open lesions directly exposed to body secretions of known cases
-Children in schools where more than one case is reported
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Hepatitis A Prohylaxis Indications
-All children and employees of child care centers where a case reported
-Custodial care residents and staff in close contact with an active case
-Persons traveling to developing countries for <3 months
-Can give HAV vaccine concurrently with IG if warranted, for those traveling internationally
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Education: Hepatitis A
-Prevention includes:
good hygiene
safe drinking water
HAV vaccine for children 12 months and older
-Children 2–18 years of age who have not received the HAV vaccine may receive the vaccine
-Good hand hygiene with toileting and diaper changes are important to prevent transmission in the home and childcare center
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Hepatitis B
-Highly contagious and preventable with vaccine administered during infancy
-Development of chronic HBV is determined by age of initial acute HBV infection and the rate of loss of HBcAg
-More than 90% of infants infected with HBV develop chronic infection
-Approximately 25–50% of children aged 1–5 years old who develop HBV infections will go on to develop chronic infection
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Transmission: Hepatitis B
-Transmitted via percutaneous or permucosal exposure, including blood, semen, vaginal secretions, cerebrospinal, and pleural fluid
-It can be transmitted via birth from an infected mother, sexual contact with an infected person, personal hygiene items such as toothbrushes or razors, and infected medical equipment
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Risk Factors: Hepatitis B
-Lack of immunization among children who have immigrated to the United States from sub-Saharan Africa and East Asia, or other areas of the world with endemic states of HBV
-IV drug users and those who engage in sexual contact with infected persons -Healthcare workers exposed to contaminated bodily fluids
-Tattooing and body piercing with infected equipment
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Clinical Manifestations: Hepatitis B
-Children who are infected with HBV at an early age are less likely to present with symptoms
-Gradual onset of nonspecific symptoms includes fever and nausea
-Hepatomegaly
-Tenderness of the liver
-Rash
-Papular acrodermatitis in infants
-Jaundice of the skin, mucous membranes, and sclera
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Indications for Hepatitis B IVIG
-Newborns who mothers are HBsAg positive
-Household contacts <12 months old who have received only one prior HBV vaccine and the second dose is not due
-Sexual contact or needle-sharing with known HBsAg positive cases, including secual assault or abuse victims
-Individuals with percutaneous or permucosal exposure to body secretions of known cases
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Management: Hepatitis B
-If a mothers HBsAg is unknown before delivery, infants should receive HBV vaccine and HBIG in 2 different sites within 12 hours of birth or 24 hours of blood exposure
-HBIG can be given within 7 days of delivery if the mother tests positive for HBsAg postpartum but its less effective
-Give HBIG and HBV vaccine up to 14 days after last exposure and repeat vaccine at 1 to 6 months for sexual partners of known cases
-In <12 months with household contacts, give HBIG and 3 doses of HBV vaccine
-Hepatitis B vaccine can be used for postexposure prophylaxis if given within 12 to 24 hours after exposure
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Management: Chronic Hepatitis B
->12 months:
interferon-alfa
->3 years:
lamivudine
->12 years:
adefovir
tenofovir
->16 years:
entecavir
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Management: Chronic Hepatitis B
-Yearly liver ultrasounds
-Liver function and a-fetoprotein concentration testing
-HAV vaccines
-HBIG and corticosteroids are not found useful for chronic Hep B
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Hepatitis C
-Hepatitis C infection causes most cases of chronic hepatitis
-About half of people infected with HCV are unaware of their infection and most who become infected with HCV develop chronic hepatitis C
-The incidence of chronic pediatric liver failure increases with the duration of infection
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Transmission: Hepatitis C
-Transmitted via blood
-The primary source of HCV infections in children is perinatal transmission, however about half of the children who become infected in this way typically see resolution between 24 months and 7 years of age
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Risk Factors: Hepatitis C
-Injection drug use
-Men who have sex with men are also at increased risk
-Vaginal births and breastfeeding do not increase the chances of HCV transmission
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Clinical Manifestations: Hepatitis C
-Most children with HCV do not exhibit any symptoms
-Older children may have flu-like symptoms, such as nausea or anorexia
-Exam findings may include jaundice and RUQ abdominal pain
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Education: Hepatitis C
-Children with HCV who take acetaminophen or antiretrovirals should be closely followed and their hepatic transaminases monitored
-Children diagnosed with HCV should still receive the HAV and HBV immunizations
-They may still attend daycare as usual
-Discourage alcohol consumption in adolescents and young adults with HCV infection because of the potential for further liver injury
-Education regarding not sharing razors and toothbrushes should be given as well
-Educate young adults about using condoms to prevent spreading the infection if they are sexually active
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Hepatitis D
-Uncommon in children
-It involves concomitant infection with Hepatitis B, as HDV requires the Hepatitis B virus to replicate
-HDV will either be a coinfection or super infection with Hepatitis B
-HDV is transmitted through mucosal, sexual, parenteral, or percutaneous contact with infected blood
-No vaccine is currently available, however, the HBV vaccine also prevents against HDV because of the comorbidity with HBV
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Risk Factors: Hepatitis D
-Drug use
-Hemophilia
-Immigration from southern Italy, some parts of Eastern Europe, South America, Africa, and the Middle East
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Hepatitis E
-It can also be found in zoonotic hosts such as swine and nonhuman primates
-It is transmitted through the fecal-oral route, most commonly via contaminated water
-Children are typically asymptomatic or mildly symptomatic
-Older adolescents and young adults are typically symptomatic
-Patients usually make a complete recovery and chronic infections are considered rare
-Mortality rate is very low but increases for pregnant females
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Risk Factors: Hepatitis E
Most cases in the United States are found in immigrants or visitors from India, the Middle East, some areas of Africa, Southeast Asia, and Mexico
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Clinical Manifestations: Hepatitis E
-Presents similarly to other hepatitis infections, and usually within 15–60 days of infection
-Malaise
-Anorexia
-Fever
-Abdominal pain
-Arthralgia
-May be jaundiced
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Management: Hepatitis E
-HEV management includes supportive management
-There is no approved vaccine.
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Education: Hepatitis E
Good hand hygeine
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HIV Pathogenesis
-HIV enters cells to target a CD4+ T cell
-Converts RNA into DNA using the reverse transcriptase enzyme
-Integrates with the T cell DNA within the cell nucleus
-Permanent infection of the host cell
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Transmission: HIV
-Found in blood, CSF, pleural fluid, cervical secretions, human milk, feces, saliva, and urine
-Transmission of the virus can occur via blood, semen, cervical secretions, and human milk
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In Utero Transmission: HIV
-This accounts for 30% of infections
-It is associated with early and severe newborn disease
-This usually occurs by 10 weeks gestation.
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Intrapartum Transmission: HIV
-This accounts for at least 60% of infections
-It results from exposure to infected blood and cervicovaginal secretions in the birth canal or due to micro transfusions between the infected mother and infant during labor
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Postpartum Transmission
-This accounts for approximately 15% of infections in the United States
-It occurs via breast milk
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Clinical Manifestations: HIV
-Flu-like symptoms:
fever
rash
sore throat
muscle pain
-Failure to thrive
-Chronic or recurrent diarrhea
-Pneumonia
-Recurrent bacterial infections in older kids
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Clinical Manifestations Continued: HIV
-Newborn exams are usually normal
-Lymphadenopathy hepatosplenomegaly
-Oral candidiasis
-Chronic parotid swelling
-Progressive neurologic deterioration
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Diagnostic Studies: HIV
-Within 48 hours
first HIV DNA PCR or HIV qualitiative RNA assay from peripheal blood and comfirm if positive using the same test on another blood sample
-Within 14 to 21 days
optional HIV DNA PCR or HIV qualitative RNA assay, confirm if positive
-1 to 3 months
second HIV DNA PCR or HIV qualitative RNA assay, confirm if positive
-4 to 6 months
third HIV DNA PCR or HIV qualitative RNA assay, confirm if positive
-12 to 24 months
fourth HIV DNA PCR or HIV qualitatitive RNA assay, confirm if positive
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Treatment Goals: HIV
-Suppression of viral replication to undetectable levels
-Restoring or preserving immune function
-Reduction of HIV-associated sequelae
-Minimization of drug toxicity
-Promotion of normal growth and development
-Promotion of treatment regimen adherence
-Improving quality of life
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Managment: HIV
-PCP’s role is to boost adherence rates and possibly monitor for side effects from drug regimen
-Factors associated with lower adherence to treatment regimen include mental health issues, age, homelessness, inconsistent access to antiretroviral therapy, and stigma
-Factors associated with greater adherence to treatment regimen include trust and satisfaction with HIV provider
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Management Continued: HIV
-Current treatment regimen recommendations are at least three oral antiretroviral drugs from at least two drug classes
-Generally 2 nucleoside reverse transcriptase inhibitors (NRTIs) plus either a non-nucleoside reverse transcriptase inhibitor (NNRTIs) or protease inhibitor (PI) often with low-dose ritonavir
-Management specifics are determined by the specialist
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Discharge management for infants with unknown HIV status
-6 week course of zidovudine (Retrovir) prophylaxis protocol for HIV-exposed newborn from birth to 6 weeks
-Ensure bloodwork is done prior to antibiotic prophylaxis
-Should be prescribed trimethoprim-sulfamethoxazole prophylactically to prevent Pneumocystis jirovecii beginning at 4–6 weeks of age through the first year of life
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Education: HIV
-Adolescents who are HIV positive should use condoms during sexual incounters
-Risk of sharing needles
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Tuberculosis
-Caused by M. tuberculosis
-Transmitted by droplet contamination from coughing, sneezing, or talking
-Droplets can stay in the air for hours
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Latent TB vs Active TB
-Latent TB:
the child is infected but not contagious
-Active TB:
the disease is present and contagious
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Risk Factors: Tuberculosis
-Immigration
-International adoptees or travelers from high-prevalence regions such as Asia, Africa, Latin America, and the former Soviet Union countries for 1 week or more
-Homelessness
-Alcoholism
-IV drug use
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Risk Factors Continued: Tuberculosis
-Persons in correctional facility
-Recent TB infection within the last two years
-Immunocompromised IV drug use
-Comorbid chronic conditions such as lymphoma and diabetes, chronic renal failure, and inflammatory bowel disease
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How long after exposure will a Mantoux Tuberculin Test be positive?
4 to 8 weeks
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When do you read a Mantoux Tuberculin Test?
48 to 72 hours
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Positive Mantoux Tuberculin Test Interpertation
-Induration (5mm or greater) in children who are:
in close contact with an individual with active or previously active TB cases
have a chest X-Ray with signs of active or previous TB
clinical findings
have immunosupprressive disorder or HIV
receiving immunosuppressive drugs
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Positive Mantoux Tuberculin Test Interpretation Continued
-Induration (10mm or greater) in children <4 years with any high risk factors
-Induration (15 mm or greater) in children >4 years without any risk factors
-If the skin test shows induration onset after 72 hours, it should be interpreted as positive
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What test should you use if a patient is >5 years and had a recent BCG vaccination?
IGRA that includes QuantiFERON TB Gold In-Tube and T-SPOT.TB
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When to Draw a Interferon Y Release Assay
-Immunocompetent children >5 years to confirm suspected LTBI or active disease
-A positive result indicates TB infection: a negative IGRA should not rule out infection or disease if clincial signs and symptoms suggest otherwise
-Children >5 years who received BCG vaccine
-Children >5 years who are unlikely to return for TST reading
-If the initial TST is positive in children >5 years who received BCG vaccine, in whom additional evidence is needed to improve compliance, or if nontuberculous mycobacterial disease is suspected
-Might be used with other diagnostic tests in an infant suspected of having congenital TB
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Clinical Manifestation: TB
-Most children ages 3–15 years with primary TB are asymptomatic
-Symptoms may present 1–6 months after the infection begins:
low-grade fever, malaise, and decreased appetite
nonproductive cough, dyspnea (in infants), night sweats, and chills
erythema nodosum, phlyctenular keratoconjunctivitis
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Clinical Manifestations Continued: TB
-Extrapulmonary symptoms can be present in approximately 25% of children:
meningitis
granulomatous inflammation of the lymph nodes, middle ear, or mastoid
worsening pulmonary infection may present as recurrent cough, stridor, and wheezing
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Hallmark Sign: TB
Disproportionately enlarged regional lymph nodes compared with a relatively small pleural focus
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Other Diagnostic Studies: TB
-TB aspirate from gastric aspirates, sputum, bronchial washings, pleural fluid, CSF, urine, body fluids, or biopsy
-Induce coughing for children >5 years and older with hypertonic saline
-If they are <5 years, early morning gastric aspirates, collected on 3 seprate mornings and analyzed by fluorescent staining
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Complications: TB
-Progressive pulmonary disease
-Miliary disease
-Pleural effusions
-TB meningitis
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Management: TB
-Consult a Pediatric TB specialist if the child has a positive TST, suspected TB, or TB contact
-First-line treatment is administered orally and includes specific regimens of isoniazid, rifampin, pyrazinamide, and ethambutol
-The primary care provider should be familiar with their patient’s specific antitubercular drug regimen and see the patient monthly to monitor their response to treatment
-PCP should monitor drug adherence and for signs of hepatitis
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Education: TB
-Primary care providers should promote strict adherence to treatment regimen to prevent drug resistance
-Educate patient and family about warning signs of hepatotoxicity while on an anti-tubercular drug regimen
-Rifampin may cause orange secretions in urine, vomiting, hepatitis, flulike symptoms, thrombocytopenia, and pruritis
-Patients on oral contraceptives will need to use a backup birth control method while on rifampin
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Vaccines and TB
Children with TB can be given measles and other attenuated live virus vaccines at age-appropriate times, unless they are on high-dose corticosteroids, are severely ill, or have another contraindication
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Clinical Manifestation: Down Syndrome
-Hypotonia in infancy
-Hearing loss
-Developmental delays
-Short stature
-Small ears
-Small mouth and protruding tongue
-Clinodactyly (finger or toe turns sideways)
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When do you refer to genetics?
-If there is a positive history for an inherited disorder for which the child is at risk
-Physical findings/dysmorphic features on physical exam consistent with a known syndrome
-Known inborn errors of metabolism
-Noted developmental, growth, and/or structural anomalies
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Clinical Manifestation: Down Syndrome Continued
-Upslanting palpebral fissures
-Epicanthal fold
-Open mouth with protruding tongue
-Brachycephaly (head is shorter than normal in the front-to-back dimension)
-Redundant skin
-Small ear with overfolded helix
-Flat facial profile
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Management: Down Syndrome
-Review newborn screen for hypothyroidism and annual review for hypothyroidism
-Evaluate routinely for critical congenital heart disease
-Monitor growth using the Down syndrome specific growth chart
-Monitor eye exams for signs of cataracts
-Monitor hearing with routine hearing evaluations
-Monitor for neurologic complications such as seizures or moyamoya
-Monitor for signs of atlantoaxial instability
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Clinical Manifestations: Achondroplasia
-Dwarfism
-Vaulting of the skull
-Frontal bossing
-Hydrocephalus
-Hypotonia
-The infant may have developmental delays as well
-Macrocephaly
-Bowing of legs
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Clinical Manifestations: Achondroplasia Continued
-Short stature
-Shortening of limbs
-Short foot and toes
-Macrocephaly
-Frontal bossing
-Depressed nasal bridge
-Characteristically short hands that have a “trident” position in extension
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Management: Achondroplasia
-Growth management using syndrome specific growth chart
-Monitor for hydrocephalus, particularly during the first two years of life when it is more likely to present
-Monitor for sleep apnea
-Follow annual hearing evaluations as middle ear dysfunction is common
-Prescribe medication dosages that are appropriate for the child’s age and size
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Education: Achondroplasia
-Educate parents/caregivers about macrocephaly and skeletal dysplasia as well as increased risk for cranio-cervical junction compression
-Advise to use rear-facing car seats
-Educate parents/caregivers about the risk of lower extremity bowing and thoracolumbar kyphosis
-Advise to avoid devices that cause curved sitting like umbrella strollers
-Advise to avoid jumpers and walkers until the child can sufficiently bear weight
-Advise to avoid living at high elevations due to restrictive pulmonary disease
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Clinical Manifestations: Marfan Syndrome
-Neurodevelopmentally normal with phenotypic variability, including variable facial features
-Facial features vary and include:
long, narrow facies
downward slanting eyes
micrognathia
-Tall for their age with long extremities that are disproportionate to their trunk
altered arm-span: height ratio
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Clinical Manifestations: Marfan Syndrome Continued
-Skeletal issues such as:
pectus deformities
scoliosis
pes planus
increased joint laxity
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Complications: Marfan Syndrome
-Aortic root dilatation
-Spontaneous pneumothorax
-Reduced pulmonary reserve
-Sleep apnea
-Recurrent hernias
-Retinal detachment
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Management: Marfan Syndrome
-The PNP should closely monitor growth using syndrome-specific growth charts
-Monitor for musculoskeletal deformities as well, including scoliosis, pectus deformities, joint laxity, and pes planus
-Ensure appropriate ongoing cardiac screening to evaluate for aortic dilatation, including an echocardiogram
-Beta blockers can be used for treatment
-Advise restricted involvement in contact and competitive sports
-Aerobic activities should be done in moderation
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Clinical Manifestations: Angelman Syndrome
-Intellectual disability
-Hand flapping
-Spontaneous persistent social smile or fits of laughter
-Speech delay with appropriate receptive language
-Abnormal sleep
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Clinical Manifestations: Angelman Syndrome Continued
-Fascination with water
-Abnormal gait with arms held high and flexed elbows
-Hypotonic trunk with hypertonic limbs
-Microcephaly
-Seizures
-Feeding and growth problems
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Management: Angelman Syndrome
-Monitor for developmental delays and speech problems
-Absent or near-absent speech
-They may not ever develop expressive speech, but can understand basic commands and older children may communicate with gestures
-Monitor for seizure activity
-Monitor for scoliosis
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Clinical Manifestations: Prader-Willi
-Hypotonia and feeding problems in infancy
-Behavioral problems: tantrums, stubborn and rigid personality, ADHD, high pain tolerance, and skin picking
-Mild intellectual disability, motor and language delays, and poor coordination
-Short stature
-Failure to thrive
-Central obesity
-Sleep apnea
-Scoliosis
-Strabismus
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Complications: Prader-Willi
-High pain threshold, which creates challenges for PCP to address illness/injury in timely manner
-Children with Prader-Willi syndrome rarely vomit
-Reduced salivation leads to early increased risk for dental caries
-Hypogonadism and premature adrenarche are common
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Management: Prader-Willi
-Monitor growth using standard growth charts
-Monitor for injury due to high pain tolerance
-Monitor for failure to thrive
-Assist families with behavioral issues including:
hyperphagia
tantrums
obsessive behavior
elopement
81
Clinical Manifestations: Fragile-X Syndrome
-Intellectual disability
-Language delays
-Behavioral problems such as anxiety and aggression
-Stereopathies
-Autism spectrum disorder
-Prominent forehead
-Long, narrow face
-Prominent jaw
82
Clinical Manifestations: Fragile-X Syndrome Continued
-High arched palate/dental crowding
-Protuberant ears
-Short stature
-Connective tissue dysplasia
-Obstructive sleep apnea
-Recurrent/chronic OM
-Seizures
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Management: Fragile-X Syndrome
-Closely monitor development
-Cognitive deficits can be moderate to severe
-Monitor for hypersensitivity to sensory stimuli
-Monitor for seizure activity as there is an increased risk for seizure (decreases in adolescence)
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Management: Fragile-X Syndrome Continued
-Evaluate infants for congenital hip dysplasia, clubfoot, and hypotonia
-They may need orthopedic referral for connective tissue dysplasia such as scoliosis or hypermobile joints
-Evaluate for inguinal hernias as children with Fragile X are at increased risk
-Ensure annual ophthalmologic evaluation
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